hypervalinemia /hī′pərval′inē′mē·ə/ , 1. an autosomal-recessive aminoacidopathy, probably caused by a defect in an enzyme necessary for valine catabolism, characterized by elevated levels of valine in the plasma and urine and by failure to thrive. 2. elevated levels of valine in the plasma. Hypervalinemia is often associated with maple sugar urine disease. Also called valinemia.